Abstract

Introduction Both primary and secondary mitochondrial respiratory chain dysfunction result in generation of abnormal levels of free radicals that deplete intracellular stores of glutathione. The glutathione system is the main natural defense mechanism that helps to combat oxidative damage. We report the intracellular reduced glutathione (iGSH) levels in patients with either primary mitochondrial disease or organic acidemias.

Conclusions T cells (CD4 and CD8 subsets), neutrophils, and monocytes may have lower iGSH levels in mitochondrial disorders and organic acidemias. In subjects taking antioxidants, there appears to be sparing of iGSH levels. The significance of the selective low iGSH levels in T cell subsets when compared to B cells is unclear. Given that T cell iGSH levels tend to be low in mitochondrial disorders and organic acidemias, it is reasonable to explore the therapeutic effect of N-acetylcysteine in these disorders.

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